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Virchows Archiv

, Volume 473, Issue 1, pp 23–31 | Cite as

Pathology of idiopathic non-cirrhotic portal hypertension

  • Maria Guido
  • Samantha Sarcognato
  • Diana Sacchi
  • Guido Colloredo
Review and Perspectives
Part of the following topical collections:
  1. Vascular liver lesions: contemporary views on long-recognized entities

Abstract

Idiopathic non-cirrhotic portal hypertension is an under-recognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis. By definition, any disorder known to cause portal hypertension in the absence of cirrhosis and any cause of chronic liver disease must be excluded to make a diagnosis of idiopathic non-cirrhotic portal hypertension. However, the diagnosis is often difficult because the disease resembles cirrhosis and there is no gold standard test. Liver biopsy is an essential tool: it is able to exclude cirrhosis and other causes of portal hypertension and it allows the identification of the characteristic lesions. Nonetheless, the histological diagnosis of idiopathic non-cirrhotic portal hypertension is not always straightforward, in particular by needle biopsy samples, because there is no pathognomonic lesion, but rather a variety of vascular changes which are unevenly distributed, very subtle, and not all necessarily identified in a single specimen. Pathologists should be able to recognize several patterns of injury, involving portal/periportal areas as well as parenchymal structures.The histological features of idiopathic non-cirrhotic portal hypertension are described in this review, focusing on their interpretation in needle biopsy specimens.

Keywords

Idiopathic non-cirrhotic portal hypertension Vascular lesions Obliterative portal venopathy Incomplete septal cirrhosis Nodular regenerative hyperplasia 

Notes

Compliance with ethical standards

This review complies with ethical standards and does not involve human participants or animals.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Maria Guido
    • 1
  • Samantha Sarcognato
    • 1
  • Diana Sacchi
    • 1
  • Guido Colloredo
    • 2
  1. 1.Surgical Pathology & Cytopathology Unit, Department of Medicine – DIMEDUniversity of PadovaPaduaItaly
  2. 2.Department of Internal Medicine, San Pietro HospitalPonte San PietroItaly

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